Informed Consent

Before you consider testosterone treatment, an operation to  remove gynaecomastia or invasive sperm retrieval, especially when the patient is a child, it is important that you read this.

The society we live in is based on the notion that everyone is either male or female. It is the first question which is asked when a baby is born – is it a boy or a girl?

The answer is usually obvious. We look at the baby’s genitalia – if it has a penis it is a boy, if not, it is a girl.  Well, no, it isn’t always that simple. Sometimes the internal reproductive organs don’t match the external ones. For example, a child with a penis may  have ovaries. What is the answer then?

In the general population babies are sometimes born with ambiguous genitalia. For example, it may be difficult to tell if the baby has an enlarged clitoris or a small penis. What is the answer then?

The answer is that not everyone fits this rigid stereotype, some can’t be classified and many don’t want to be. There is now a growing awareness in society as a whole that gender identity isn’t two fixed points but, instead, is a spectrum – some males are more ‘male’ than others and some females are more ‘male’ than others. Most people’s gender matches their sex, but for some the two don’t agree. Some people are very clear as to what gender they are, others less so. Some may feel ‘gender fluid’.

Only the individual knows whether they feel male, female, neither or both regardless of how they appear to others. This applies to XXY people too. Most are male, a few are female and others are neither – or both.
It is perhaps a little more complicated for XXY folk because, although most XXY babies appear to be male, their chromosome pattern (47, XXY)  isn’t male (46,XY) or female (46, XX).  KS is classified as a Disorder of Sexual Development (DSD) although many prefer the older term, Intersex. The KSA prefers the usage Variation of Sexual Development (VSD).

After puberty XXY children may develop some female characteristics such as breast formation and a lack of body hair. Some youngsters may be very unhappy if they appear less male than their peers, others may not. Some may want to have an operation to remove any breast tissue to make them appear more ‘male’ – others may not be concerned. Some may really want to have testosterone treatment – others may not.

The important thing to remember is that, ultimately, only the young person can make these decisions. It has to be their decision as to whether or not they want treatments or procedures which aren’t medically required but which may help them to conform or feel better about themselves The youngster is the only one who knows how it feels, who knows how it wants to be identified – him, her, it, they.

Hormone treatment doesn’t just alter the body. It also has an effect on personality and identity. Testosterone causes a person to develop ‘male’ characteristics such as facial hair, increased assertiveness and higher libido – which is fine for someone who wants to be more ‘male’ but may not be helpful for someone who is happy the way they are, who values their differences and who doesn’t want to be changed. There are still others who wish to be more ‘female’.

These treatments are life changing and these decisions should not be made until the young person has the capacity to make them themselves. Parents and medical professionals should not put pressure on the child and, regardless of their own feelings, parents should make it clear to their child that they will support them whatever their choice.

It is important that it is recognised that although  testosterone replacement may suit many, it isn’t the best option for everyone. There is no ‘correct’ decision – it is down to the individual and they must be allowed to make the decision free of external pressure. Some level of gender assessment or counselling may be useful.

Gender is different from sexual orientation or sex. Those with KS have intersex bodies but may be male, female, neither or both and their sexual orientation may be straight, gay or both– just like anyone else. There is no research to indicate that being XXY increases the incidence of being gay and there is only anecdotal evidence to suggest that the incidence of gender fluidness may be higher in the XXY community.

In such a sensitive and complex area, parents may be in doubt about how best to support their children in this and they should be encouraged to seek the support from counselling or psychological services. Professionals and parents should adopt a team approach, providing a circle of support for the individual.

Next… Prognosis